Angervall L, Kindblom L G, Lindholm K, Eriksson S
Department of Pathology, Gothenburg University, Sahlgren Hospital, Sweden.
Pathol Res Pract. 1992 Apr;188(3):350-6; discussion 356-9. doi: 10.1016/S0344-0338(11)81216-1.
A typical case of plexiform fibrohistiocytic tumor (Enzinger and Zhang) occurring in the skin and subcutis of the abdominal wall in a 7-year-old girl is reported. Preoperative fine-needle aspiration cytology revealed a benign lesion with fibroblastic-histiocytic features which also contained bi- and multinucleated giant cells. The surgical specimen showed a tumor with multiple small nodules within fibrous septa; these nodules were composed of spindle cells and epithelioid cells and contained scattered multinucleated osteoclast-like cells. The tumor cells showed ultrastructural and immunohistochemical features of myofibroblasts and histiocyte-like cells. Thus, there was an abundance of lysosomes, prominent filopodia and bundles of thin cytofilaments along the cytoplasmic border, as well as immunoreactivity for alpha-smooth-muscle-specific actin, alpha-1-antitrypsin and alpha-1-antichymotrypsin. Ultrastructurally there were tumor cells exhibiting features of histiocytes which also contained bundles of actin of smooth muscle type. The presented case of plexiform fibrohistiocytic tumor appears to be composed of a rather peculiar cell form, somewhere between myofibroblasts and histiocytes.
报道了1例发生于7岁女童腹壁皮肤及皮下组织的典型丛状纤维组织细胞瘤（恩津格和张）。术前细针穿刺细胞学检查显示为具有成纤维细胞-组织细胞特征的良性病变，其中还含有双核和多核巨细胞。手术标本显示肿瘤在纤维间隔内有多个小结节；这些结节由梭形细胞和上皮样细胞组成，并含有散在的多核破骨细胞样细胞。肿瘤细胞显示出肌成纤维细胞和组织细胞样细胞的超微结构及免疫组化特征。因此，有大量溶酶体、明显的丝状伪足和沿细胞质边缘的细细胞丝束，以及对α-平滑肌特异性肌动蛋白、α-1抗胰蛋白酶和α-1抗糜蛋白酶的免疫反应性。超微结构上，有表现出组织细胞特征的肿瘤细胞，其中也含有平滑肌型肌动蛋白束。所报道的丛状纤维组织细胞瘤病例似乎由一种相当特殊的细胞形式组成，介于肌成纤维细胞和组织细胞之间。
